Management of hereditary angioedema: a Canadian approach.

C1 esterase inhibitor (C1-INH) deficiency is a rare disorder that lacks consensus for diagnosis therapy and management. Recognizing that Canada is behind the European approach to this disorder, we have formed the Canadian Hereditary Angioedema Society (CHAES)/Société d'angioédème héréditaire du Canada (SAHC) to foster knowledge of this disorder in Canada and to advance care of patients with this disorder in Canada. We here present a review of treatment of this disorder in Canada including prevention of angioedema events and use of replacement therapy and present an algorithm for diagnosis therapy and management of C1-INH deficiency in Canada for discussion at our International Conference on Hereditary Angioedema to be held in Toronto, Canada, October 24th to 26th, 2003.     

The clinical diagnosis of ventilator-associated pneumonia

There has long been a controversy about whether to use a clinical or microbiologic approach to diagnose ventilator-associated pneumonia (VAP) and about which approach to use in managing patients. Although the clinical approach has often been criticized, a number of recent studies have shown that it is possible to use such an approach to effectively manage patients. This approach involves using all available clinical data to define the presence of pneumonia and then to initiate empiric therapy in a timely fashion, based on therapy guidelines, modified by local microbiologic data. Often the clinical diagnosis is made using the clinical pulmonary infection score, and this tool can be very accurate, especially if it incorporates a Gram stain of a lower-respiratory-tract sample. Once the clinical diagnosis of VAP is made, all patients should have a tracheal aspirate collected for culture, followed by prompt initiation of therapy. Using a clinical approach to management, the key decision point is not whether to start antibiotics, but whether to continue them at day 2-3. This requires serial clinical evaluation to define whether a response to empiric therapy has occurred. Based on this assessment, in conjunction with the results of tracheal aspirate cultures, therapy can be either modified or continued. A number of studies have shown that the clinical approach leads to a large number of patients receiving adequate empiric therapy, while still permitting de-escalation of antibiotic regimens, along with short durations of therapy. Thus a clinical approach to management can be successful in allowing for effective management of VAP, without promoting the unnecessary use of broad-spectrum antimicrobial therapy.     

Evidence-Based Approach to the Physical Therapy Diagnosis and Management of Neck and Upper Extremity Pain using Cervical and Thoracic Spine Thrust Manipulation: A Case Report

Abstract

Neck and upper extremity pain are common medical diagnoses for patients seeking physical therapy care. The purpose of this case report is to describe an evidence-based approach to the physical therapy diagnosis and management of a 46-year-old female reporting insidious onset neck pain and bilateral upper extremity paraesthesiae of two years duration. Evaluation of examination data, based on research data with regard to diagnostic accuracy of the tests and measures used, indicated a diagnosis of cervical radiculopathy. Management was based on a treatment-based classification approach and focused on restoring mobility by way of thrust manipulations directed at the thoracic and cervical spine. At the completion of the physical therapy plan of care (8 visits), the patient rated her perceived improvement on the Global Rating of Change Scale as "a very great deal better." The Numerical Pain Rating Score improved from 6/10 to 0/10. Patient-perceived disability, as measured by the Neck Disability Index, improved from 26% to 0%, and the patient's score on the modified Oswestry Disability Index improved from 30% to 0%. Bilateral upper extremity paraesthesiae also had completely resolved. These clinically meaningful improvements in pain and perceived disability were maintained six weeks after discharge. While a cause-and-effect relationship cannot be inferred from a case report, it is plausible that an orthopaedic manual physical therapy approach in the management of patients with both neck and upper extremity pain may result in decreased pain and improved function. Further clinical trials are needed to test this hypothesis.     

Rhythm disturbances

Patients with cardiac rhythm disturbances may present in a variety of conditions. Patients may be unstable, requiring immediate interventions, or stable, allowing for a more deliberate approach. Rapid assessment of patient stability, underlying rhythm, and determination of appropriate interventions guides timely therapy. This article discusses the differential diagnosis and treatment of adult patients presenting with primary bradyarrhythmias and tachyarrhythmias, with the exception of atrial fibrillation and atrial flutter, covered elsewhere in this issue. A concise approach to diagnosis and determination of appropriate therapy is presented.     

Molecular diagnosis of leukemia

The diagnosis of leukemia relies upon a multiparametric approach involving a number of different pathology disciplines. Molecular methods are increasingly employed to help refine diagnosis, establish prognosis and determine the most appropriate treatment, including rational therapies targeting the underlying genetic lesion. This review aims to highlight some of the molecular techniques commonly used in the diagnosis of leukemia using relevant examples. The focus is on procedures in current use and technologies showing promise in the research setting that are likely to enter clinical use in the near future. The list is not exhaustive, and this article concentrates on diagnosis of leukemia; techniques used to monitor response to therapy and molecular residual disease are mentioned but have not been covered extensively.     

Management and treatment of patients with acromegaly.

Untreated acromegaly causes substantial morbidity and mortality, and results in a marked reduction in quality of life. For this reason, the treatment goals need to be well defined and therapy optimised. This article describes the clinical approach to acromegaly, including: Pathophysiology of growth hormone secretion; Clinical features; Biochemical and radiological diagnosis; Treatment goals and therapeutic options; Prognosis.     

Recent advance in inhaled corticosteroid therapy]

Inhaled corticosteroid therapy against bronchial asthma has developed rapidly and remarkably during last 10 years. The significance of this therapy has been established in a lot of international guidelines for asthma management. Early intervention, which developed from the concept of step-down therapy, is new therapeutic approach to prevent the remodeling of airway walls deemed as the major cause of irreversible airflow limitation which makes patient chronic and refractory asthmatics. It is comprehensive therapeutic approach which include not only early diagnosis, early intensive corticosteroid therapy, but also environment intervention and enough patient education. In addition to this new therapeutic strategy, we will describe about add-on effect of long-acting beta-2 agonists, theophylline, and leukotriene receptor antagonist.     

An approach to the patient with severe hypokalaemia: the potassium quiz

The objective of this teaching session with Professor McCance is to develop an approach to the management of patients with a very low plasma potassium (K(+)) concentration (P(K)). The session begins with a quiz based on six recent medical consultations for a P(K) < 2 mmol/l. Professor McCance outlined how he would proceed with his diagnosis and therapy, using the synopsis that described each patient. This approach was then applied to a new patient, a 69-year-old woman who had a large volume of dependent oedema and developed a severe degree of weakness and hypokalaemia during more aggressive diuretic therapy that included a K(+)-sparing diuretic. The initial challenge for Professor McCance was to deduce why the K(+)-sparing diuretic was not effective in this patient. He also needed to explain why the P(K) was so low on admission.     

支气管内曲霉球的临床特征分析*

目的支气管曲霉球是一种新近定义少见的肺曲霉病,探讨该类型肺曲菌病的临床特点及诊疗策略,以期通过该研究提高对该病的认识水平。方法该研究通过确诊2例支气管内曲霉球及对文献发表的29例患者临床表现、影像特点、气管镜表现、诊断及治疗等进行回顾性分析。结果该疾病常见表现有咯血、咳嗽、咳痰,影像学多表现结节或实变影,气管镜表现:主要表现新生物及坏死物阻塞支气管腔,可伴有支气管内实质损伤(和/或)空腔性病变。该病诊断是经气管镜见管腔内新生的肿物或坏死组织,活检物组织病理发现特征性菌丝即可确诊。结论支气管内曲菌球是肺曲霉菌病的少见类型,气管镜是其诊断及治疗的关键措施,对于当抗真菌治疗无效、肺内病变无吸收甚至增大时,应警惕合并肺部肿瘤的可能。     

Doppler echocardiography: application to the assessment of successful thrombolysis of prosthetic valve thrombosis

Prosthetic valve thrombosis remains a significant problem despite anticoagulation therapy and advances in valve design. Thrombolytic therapy offers an alternative approach to valve replacement in patients with high surgical risk. In this article we discuss three cases in which Doppler echocardiography was used to confirm the diagnosis of prosthetic mitral valve obstruction and serially monitor the response of valvular hemodynamic measurements to thrombolysis with intravenous streptokinase. These cases illustrate how the Doppler technique, in addition to allowing the noninvasive diagnosis of prosthetic valve obstruction, is presently the ideal tool to follow serially the effect of thrombolytic therapy on prosthetic valve function.     

Pseudomonas aeruginosa treatment and transmission reduction

Infections caused by Pseudomonas aeruginosa can be difficult to treat and require a coordinated approach for their management. This involves quickly controlling the source of infection, establishing a correct etiologic diagnosis and administering appropriate empiric antimicrobial therapy. Once antimicrobial therapy has been initiated and susceptibilities are available, therapy should be tailored with optimized antibiotic doses for an appropriate duration in order to sufficiently treat the infection and minimize resistance emergence.     

The method of teaching therapy--the problem approach to diagnosis

The methodology of a so-called task force approach to the diagnosis is suggested. It determines the strategy of the physician's diagnostic searches in some problems occurring in the examination of patients afflicted with the given disease. The problems (stages, levels of examination) show how one should reason, i. e. they form concrete bases for clinical thinking. According to this methodology, the symptoms should be classified with separate diagnostic problems--trends or stages of the patient's examination at the stage of a probable diagnosis establishment. The task force approach is a method of analysis which prevents potential gross diagnostic errors by means of deciding the main questions (functional, pathogenetic, etc.). A well-defined wording of the above-indicated problems--concrete regularities of the clinical thinking--are of paramount importance in teaching therapy. Examples are provided of the task force approach to the diagnosis of pyelonephritis, infectious myocarditis, anemia, and some other diseases.     

Diagnostic and therapeutic approach to the patient with vasculitis

This article presents an approach to the diagnosis and therapy of patients with vasculitis. Effective treatment of patients with vasculitis requires a systemic approach to diagnosis and classification of disease, evaluation of the extent of organ system involvement, and an exclusion of underlying treatable diseases.     

Changing the Goal Posts: Prostate-specific Membrane Antigen Targeted Theranostics in Prostate Cancer

ObjectiveProstate-specific membrane antigen (PSMA) theranostics is changing the face of prostate cancer diagnosis and therapy. PSMA, a transmembrane protein over-expressed in many prostate cancers, is a promising target for theranostics. Theranostics is the concept of small molecule proteins that are labelled to different radionuclides and can be used for either diagnosis or therapy, dependent on whether they are labelled with an imaging or therapy radionuclide. By directly targeting the cancer cells with imaging and then for therapy, this approach embodies the philosophy of precision medicine – right drug, right time, right dose. The question is how to best utilise these new imaging and therapy agents in clinical practice. This review will evaluate the importance of PSMA in prostate cancer, its role in diagnostic imaging, and its potential as a therapy of advanced prostate cancer.Data SourcesElectronic databases including MEDLINE, Scopus, professional websites were searched.ConclusionPSMA-directed theranostics has an expanding role in prostate cancer because of its utility as a sensitive diagnostic tool that can be coupled with efficacious and low-toxicity therapeutic options. Ongoing research is required to determine how to use this effective tool for best patient care.Implications for Nursing PracticePSMA theranostics is rapidly being incorporated into the routine care of men with prostate cancer. Understanding its strengths, its limitations, and where it may be valuable in clinical care is important in undertaking best patient practice.     

Solutions for difficult diagnostic cases of community-acquired pneumonia

Community-acquired pneumonia (CAP) is a common illness that creates significant burdens upon the healthcare system. Improving the quality of medical care for patients with this illness requires an evidence-based and cost-efficient treatment approach. The first step in this approach is to make an accurate diagnosis, while considering the full differential diagnosis of the illness. This requires an understanding of the sensitivity and specificity of the history and physical examination to establish the diagnosis of CAP. The second step is to quantify severity of illness, which can help physicians determine the appropriate initial site of treatment, intensity of the diagnostic evaluation, and choice of initial antibiotic therapy. Case histories are presented to outline the clinical application of an approach that uses the Pneumonia Patient Outcomes Research Team (PORT) prediction rule for prognosis to quantify the severity of illness, and recent guidelines for the management of CAP are highlighted.     

Use of an artificial neural network to predict Graves' disease outcome within 2 years of drug withdrawal

BACKGROUND: Graves' disease (GD) is an autoimmune disorder characterized by hyperthyroidism, which can relapse in many patients after antithyroid drug treatment withdrawal. Several studies have been performed to predict the clinical course of GD in patients treated with antithyroid drugs, without conclusive results. The aim of this study was to define a set of easily achievable variables able to predict, as early as possible, the clinical outcome of GD after antithyroid therapy. METHODS: We studied 71 patients with GD treated with methimazole for 18 months: 27 of them achieved stable remission for at least 2 years after methimazole therapy withdrawal, whereas 44 patients relapsed. We used for the first time a perceptron-like artificial neural network (ANN) approach to predict remission or relapse after methimazole withdrawal. Twenty-seven variables obtained at diagnosis or during treatment were considered. RESULTS: Among different combinations, we identified an optimal set of seven variables available at the time of diagnosis, whose combination was useful to efficiently predict the outcome of the disease following therapy withdrawal in approximately 80% of cases. This set consists of the following variables: heart rate, presence of thyroid bruits, psycological symptoms requiring psychotropic drugs, serum TGAb and fT4 levels at presentation, thyroid-ultrasonography findings and cigarette smoking. CONCLUSIONS: This study reveals that perceptron-like ANN is potentially a useful approach for GD-management in choosing the most appropriate therapy schedule at the time of diagnosis.     

Use of thoracic spine manipulation in the treatment of adhesive capsulitis: a case report

Abstract

Adhesive capsulitis (AC) is a common and disabling shoulder condition seen in physical therapy, and there is no clear consensus as to the best treatment approach. Recently there has been emerging evidence that manual therapy directed at the thoracic spine may be beneficial for patients with shoulder pain; however, this has not been examined specifically in patients with AC. The purpose of this paper is to present the case of 59-year-old female referred to physical therapy with a diagnosis of AC. The patient presented with complaints of left shoulder pain and significant limitations in range of motion (ROM) and upper extremity function. The initial treatment included exercises and manual therapy directed at the glenohumeral and scapulothoracic joints, and after 10 visits only minimal progress had been made. Further examination revealed mobility and ROM deficits in the thoracic spine, and manual therapy directed at this region was incorporated into her treatment. After the first session of thoracic spine manual therapy (TSMT) a 25 degree improvement was noted in active shoulder flexion. After four total visits of TSMT substantial improvements in pain, ROM, and function were noted compared to those made during the first 10 visits. This case adds to the emerging evidence that manual therapy directed at the thoracic spine should be considered for patients with shoulder pain.     

Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to accumulation in the alveoli of proteinaceous material rich in surfactant protein and its component. Novel insights from an animal model aided the discovery of granulocyte macrophage colony stimulating factor (GM-CSF) antibodies as a pathogenetic mechanism in human pulmonary alveolar proteinosis. The vast majority of pulmonary alveolar proteinosis occurs as an autoimmune disease; less commonly, it is congenital or secondary to an underlying disorder such as infection, hematological malignancy, or immunodeficiency. The subacute indolent course of this disease often delays the diagnosis by months to years. Crazy-paving appearance in a geographic distribution is a characteristic feature of this disease visible on high-resolution computed tomography (CT). A definitive diagnosis, however, requires lung biopsy, which typically shows partial or complete filling of alveoli with periodic-acid-Schiff-positive granular and eosinophilic material in preserved alveolar architecture. Patients with minimal symptoms are managed conservatively, whereas patients with hypoxemia require a more aggressive approach. Whole-lung lavage is the most widely accepted therapy for symptomatic pulmonary alveolar proteinosis. Correction of GM-CSF deficiency with exogenous GM-CSF is an alternative therapy. The combination of a systemic treatment (GM-CSF) and a local treatment (whole-lung lavage) augmenting the action of one another is a promising new approach. As the knowledge about this rare disease increases, the role of novel therapies is likely to be better defined and optimized.     

Hypercalcemia

The approach to the patient with hypercalcemia requires a solid understanding of the principles of calcium homeostasis, knowledge of its differential diagnosis and the associated pathophysiologies, and the clinical judgment to know when and how to administer appropriate therapy. Recent advances in our understanding of the regulation of mineral metabolism have led to new insights into these areas and have improved our ability to deal with this rather common clinical entity. The purpose of this monograph is to give a current view of hypercalcemia as it is appreciated in the outpatient and the inpatient setting. It is designed to focus the evaluation of the hypercalcemic patient in a way that is both expedient and accurate as well as to take advantage of available therapies. A summary of the underlying physiological principles of calcium homeostasis is followed by a consideration of the complete differential diagnosis of hypercalcemia. The two most important causes of hypercalcemia, primary hyperparathyroidism and malignancy, are emphasized. Signs and symptoms of hypercalcemia are discussed with particular attention to those factors that are influential in accentuating or masking these features. Finally, an approach to the therapy of hypercalcemia is presented within a pathophysiological framework.     

Contractile dysfunction of the shoulder (rotator cuff tendinopathy): an overview

It is now over a decade since the features defining a contractile dysfunction of the shoulder were first reported. Since this time, some progress has been made to better understand this mechanical syndrome. In response to these developments, this narrative review will explore current understanding in relation to pathology, diagnosis, treatment, and prognosis of this syndrome with reference to literature specifically relating to contractile dysfunction but also literature relating to rotator cuff tendinopathy where necessary. The review not only identifies the strengths of the mechanical diagnosis and therapy approach with reference to a contractile dysfunction of the shoulder but also identifies where further progress needs to be made.